Introduction: Huntingtońs disease (HD) is a genetically determined, rare neurodegenerative disorder with pathological motor, behavioral and cognitive neurological characteristics. In light of the lack of disease-modifying therapies, stereotactically assisted intracranial therapies targeting the pallidal area (globus pallidus) and the frontostriatal circuits, such as deep-brain stimulation (DBS), stereotactic lesioning (pallidotomy), and brain-grafting approaches have gained increased recognition for the treatment of Huntington’s disease-associated motor symptoms. Material and Methods: We conducted a narrative review of in-human studies targeting the globus pallidus and adjunct structures (nucleus caudatus and putamen) using stereotactically guided intracranial approaches, such as ablative procedures (pallidotomy) and deep-brain stimulation of the internal/external globus pallidus, as well as intracranial brain-graft injection. We assessed the terms Huntingtońs disease, chorea, parkinsonism, dystonia, globus pallidus externus/internus, deep brain stimulation, lesioning, ablation, pallidotomy, brain grafts, fetal neural grafts, unified Huntingtońs disease rating scale (UHDRS), safety, efficacy and quality of life in data sources derived through searches of PubMed, Ovid MEDLINE, and Scopus, and through manual searches of the bibliographies of known primary and review articles covering the period 1990-2021. Results: Stereotactic lesioning (pallidotomy) was performed on a few HD patients (70 subjects). The outcome of this approach is similar to brain-grafting strategies, with inconsistent findings and mild-to-moderate clinical responsiveness. We also identified a total of 19 in-human DBS trials targeting the globus pallidus internus/externus for HD-associated motor symptoms (chorea, dystonia, and parkinsonism). The majority comprised case reports, along with a few prospective observational cohort studies. At the end of the average observation period, we did not detect any significant changes in the UHDRS total score after pallidotomy and restorative injections, while in contrast, the use of deep-brain stimulation resulted in a significant reduction in chorea symptoms, with the global UHDRS remaining unchanged. Conclusions: Currently, stereotactically guided treatment strategies (DBS, pallidotomy, and brain grafts) should be considered in cases where selective HD-related treatment resistance (chorea, dystonia, and parkinsonism/hyperkinesia) is present. In contrast to the case for PD, the experience of using DBS for HD is currently very limited, as the majority of published trials consist of uncontrolled observational cohort studies. The comparison of UHDRS scores pre- and post-pallidal DBS in a large proportion of HD patients demonstrated the meaningful impact of DBS on chorea symptoms without any worsening of the overall UHDRS score, due, for example, to an increase in bradykinesia.